Pleomorphic spindle cell sarcoma (PSCS) formerly known as malignant fibrous histiocytoma (MFH): a complex malignant soft-tissue tumor

A presentation defining the nature, characteristics, causation, treatment and outcome of patients with lesions formerly known as malignant fibrous histiocytoma and now as pleomorphic spindle cell sarcoma is clearly a very difficult subject. Many authors do not believe that the tumor exists and instead describe them as forms of fibrosarcomas, fibromyxoid lesions, dedifferentiated chondrosarcomas or even leiomyosarcomas. The reasons for this confusion are presumably related to the fact that the malignant pleomorphic spindle cell sarcoma does not seem to be a distinct type of lesion with specific histologic and genetic characteristics. Instead, the tumor has at least four separate histologic variations and no specific gene signature and in fact does not seem to be either familial or ethnic in presentation. In view of the fact that the tumor was traditionally the most frequently encountered malignant soft-tissue neoplasm, the world of orthopedic oncology is clearly distressed by the problems that these patients have and is joined by the radiation oncologists and chemotherapists in seeking new solutions

Extremity Soft Tissue Sarcoma in Adults

When treating soft tissue sarcomas (STSs) of the extremities, the major therapeutic goals are survival, local tumor control, optimal function, and minimal morbidity. Surgical resection of the primary tumor is the essential component of treatment for virtually all patients. A wide surgical margin is necessary for local tumor control when surgery is used without radiation, i.e., the cut should traverse normal tissue outside the reactive tumor zone. This is because sarcomas tend to infiltrate normal tissue adjacent to the evident lesion. Thus, removal of the gross lesion by a simple excision alone (only a narrow margin) is followed by relatively high rates of local recurrence. Radical resections are associated with a reduction in the local recurrence rate, but they may compromise limb function. The combination of function-sparing surgery and radiation achieves better rates of local control than either treatment alone, for nearly all patients with STSs, although combined treatment can be associated with acute wound complications in some patients and late normal tissue complications in others. Because both surgical and radiation techniques are both critically important for optimizing local control of tumor and functional outcome, it is important to manage these patients in dedicated multispecialty clinics comprised of physicians with expertise in sarcomas, including orthopedic and general oncology surgeons, radiation oncologists, medical oncologists, sarcoma pathologists, and bone and soft tissue diagnostic radiologists. Radiation therapy can be given by external beam radiation (EBRT) or brachytherapy or combination thereof. EBRT can be given either pre-operatively or post-operatively